Angiomatosis, diffuse corticomeningeal Q85.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Divry van Bogaert syndrome

History
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van Bogaert and Divry, 1945

Definition
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Corticomeningeal angiomatosis with progressive, severe neurological defects (epilepsy) and cutis marmorata

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Etiopathogenesis
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Autosomal recessive inheritance with family clustering.

Manifestation
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Congenital, in infancy or early childhood.

Clinical features
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  • Integument: Congenital Cutis marmorata (see below Livedo reticularis) on back, flanks, buttocks and legs. Acrocyanosis on hands, elbows and knees. Spotted hyperpigmentation on the trunk.
  • CNS: non-calcifying corticomeningeal angiomatosis (especially occipital) with epilepsy, progressive dementia, pyramidal and extrapyramidal movement disorders, hemianopsia and retinal angiomas.

Literature
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  1. Bussone G et al (1984) Divry-Van Bogaert syndrome. Clinical and ultrastructural findings. Arch Neurol 41: 560-562
  2. De A Aquino Gondim F et al (2003) Intracerebral hemorrhage associated with Sneddon's syndrome: ischemia-related angiogenesis the cause? Case report and review of the literature. Neuroradiology 45: 368-372
  3. Stone J et al (2001) Divry-Van Bogaert syndrome in a female: relationship to Sneddon's syndrome and radiographic appearances. Neuroradiology 43: 562-564
  4. van Bogaert L, Divry P (1945) Sur une maladie familiale caractérisée par une angiomatose diffuse cortico-méningée et une démyélinisation de la substance blanche du centra ovale. Bruxelles médical 25: 1090-1091

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Last updated on: 29.10.2020